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目的:探讨腹盆腔横纹肌肉瘤(RMS)的影像学特征及临床病理学特点。方法:回顾性分析经手术病理证实的3例腹盆腔RMS患者的CT、MR表现及临床资料,并结合文献进行总结。结果:临床表现有腹部肿块、腹痛等症状;CT平扫3例均见腹盆腔内巨大混合密度肿块,周围组织器官受压移位;MRI检查示盆腔内巨大的团块状异常信号影,信号不均;增强后不均匀轻中度强化,以周边明显,动脉期病灶内可见较多增粗、扭曲的供血动脉影,延迟期斑片状持续性强化。镜下所见肿块由弥漫异型细胞构成,呈圆形或梭形,疏密不均,核深染,易见核分裂;免疫组化平滑肌肌动蛋白(SMA)、肌原调节蛋白(MYOD)、CD99均阳性,神经元特异性烯醇化酶(NSE)、白细胞共同抗原(LCA)、细胞角蛋白(AE1/AE3)均阴性。病理诊断:胚胎性RMS。结论:RMS是一种好发于儿童的间充质源性恶性肿瘤,腹盆腔少见,影像学表现有一定特征性,确诊需要依据病理和免疫组化。
Objective: To investigate the imaging features and clinicopathological features of rhabdomyosarcoma of the abdomen. Methods: The CT, MR findings and clinical data of 3 patients with peritoneal RMS confirmed by surgery and pathology were retrospectively analyzed and summarized in the literature. Results: The clinical manifestations of abdominal mass, abdominal pain and other symptoms; 3 cases of CT scan were seen within the abdomen mixed massive mass, the surrounding tissue and organ pressure shift; MRI showed pelvic mass abnormal signal, the signal Uneven; enhanced non-uniform mild to moderate enhancement to the surrounding obvious arterial phase lesions can be seen more thickening, twisted blood supply artery shadow, delayed persistence patch enhancement. Microscopic appearance of lumps formed by diffuse abnormal cells, round or fusiform, uneven density, nuclear deep staining, easy to see nuclear fission; immunohistochemistry smooth muscle actin (SMA), myogen regulatory protein (MYOD) CD99 were positive, neuron-specific enolase (NSE), leukocyte common antigen (LCA), cytokeratin (AE1 / AE3) were negative. Pathological diagnosis: embryonic RMS. Conclusion: RMS is a malignant tumor of mesenchymal origin in children. Abdominal pelvis is rare, and imaging findings have certain characteristics. The diagnosis is based on pathology and immunohistochemistry.